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Odontodysplasia, gingival manifestations, and accompanying abnormalities
Odontodysplasia, gingival manifestations, and accompanying abnormalities K e r s i B. F a n i b u n d a , M D S , F D S R C S , a and J a m e s V. S o a m e s , PhD, F D S R C P S , F R C P a t h , b N e w c a s t l e u p o n Tyne, U.K. THE DEPARTMENT OF ORAL SURGERY AND THE DEPARTMENT OF ORAL PATHOLOGY, UNIVERSITY OF NEWCASTLE UPON TYNE
Regional odontodysplasia is an uncommon developmental dental disorder that may occasionally be accompanied by other abnormalities. A case is described in which the chief report was of a gingival enlargement arising in a female patient who also had dolichocephaly, thin calvarium, clinodactyly and transverse grooving of her fingernails, and a history of abnormal hair. Previously suggested etiologic factors and cases reported in association with other abnormalities are reviewed. (ORALSURGORALMED ORALPATHOLORALRADIOLENDOD1996;81:84-8)
R e g i o n a l o d o n t o d y s p l a s i a is a relatively u n c o m m o n c o n d i t i o n that u s u a l l y affects b o t h p r i m a r y and perm a n e n t dentitions. It occurs m o r e c o m m o n l y in f e m a l e patients and frequently i n v o l v e s the m a x i l l a r y teeth. 1 Clinically, the teeth a p p e a r discolored, h y p o plastic, and m a l f o r m e d , and the patient u s u a l l y has p a i n and s w e l l i n g c a u s e d b y p u l p a l and p e r i a p i c a l infection. R a d i o g r a p h i c a l l y , the teeth present a " g h o s t l i k e " i m a g e b e c a u s e o f the increased r a d i o l u c e n c y and thinness o f the e n a m e l and dentin and a w i d e pulp chamber. M i c r o s c o p i c a l l y , h y p o p l a s i a and h y p o m i n eralization are seen within the e n a m e l with large int e r g l o b u l a r areas in the dentin. The dental follicle m a y contain irregular calcifications. To date a p p r o x i m a t e l y 125 cases h a v e b e e n r e p o r t e d , and the dental features are w e l l d o c u m e n t e d . H o w e v e r , the cause is u n k n o w n , although several suggestions have b e e n a d v a n c e d . 2-5 This article reports an unusual case o f r e g i o n a l odo n t o d y s p l a s i a in w h i c h g i n g i v a l e n l a r g e m e n t was the presenting feature rather than the m o r e c o m m o n l y r e p o r t e d s y m p t o m s o f dental infection. CASE REPORT A 17-year-old white girl was referred by her dental practitioner for attention of a swelling of unknown duration situated in the left lower anterior region of her mouth. On general observation the patient was underweight for her age; she weighed 104 pounds (mean for age, 124.6 pounds6). Her height was within standard limits. On intraoral examination a gingival enlargement was noted on
aSenior Lecturer and Honorary Consultant, Department of Oral Surgery. bprofessor of Oral Pathology and Honorary Consultant, Newcastle upon Tyne, U.K. Department of Oral Pathology. Received for publication Feb. 15, 1995; returned for revision April 19, 1995; accepted for publication June 19, 1995. Copyright 9 1996 by Mosby-Year Book, Inc. 1079-2104/96/$5.00 + 0 7/14/67431 84
Fig. 1. Intraoral view of left lower quadrant showing discolored hypopiastic teeth accompanied by gingival enlargement. the labial and lingual aspects of the lower incisor to premolar region on the left side (Fig. t). The left lower central incisor, canine, and first premolar were discolored, pitted, and hypoplastic and gave the appearance of partial eruption because of the surrounding gingival enlargement. The lateral incisor in that quadrant was absent. The left lower first molar and the lower central incisor, lateral incisor, both premolars, and second molar on the right side were also affected by pitting and hypoplasia. The left lower second premolar and second molar were unaffected. Her fingers showed clinodactyly with ulnar or radial deviation of most digits. The nails on all her fingers had an increased curvature and exhibited transverse grooving (Fig. 2), which had been present throughout the patient's life. The nails on her toes were unaffected. Her stepparents reported that when the patient was adopted at 8 years of age, her hair was sparse, thin, and dull in color. However, the appearance had improved over the years. No other cranial or facial anomalies or abnormalities elsewhere were identifiable to link the presenting features with any syndrome. Because the patient was adopted, it was not possible to obtain a family
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Fig. 2. Nails demonstrate increased curvature and transverse grooving.
history, a history of the state of her deciduous teeth, or a history of any early birthmarks that may have disappeared later. Radiographic examination of the left lower central incisor, canine, first premolar, and the right lower central and lateral incisors revealed a reduced enamel and dentin thickness and wide pulp chambers (Fig. 3). No bony pathosis was seen associated with the roots of the teeth in the region of the swelling. Evidence of hypoplasia but not wide pulp chambers was also confirmed in the remaining clinically affected teeth mentioned previously. The root lengths of the left lower central incisor, canine, and first premolar were reduced. The unerupted right lower third molar showed features of "mulberry cusps." A lateral skull view demonstrated dolichocephaly and a relatively thinner than standard thickness of the cortical margins of the calvarium together with a pronounced parieto-occipital suture line (Fig. 4). In view of the presenting symptoms, the gingival enlargement was trimmed under local analgesia. Histopathologic examination showed chronically inflamed hyperplastic gingiva deep to which were numerous aggregates of rich cellular tissue containing multiple loci of basophilic amorphous or lamellated calcification (Fig. 5), Some of the lamellated calcifications were surrounded by a hyaline stroma with diffuse areas of calcification or were intimately associated with plump, fibroblast-like cells. Rests of odontogenic epithelium were present within the foci and surrounding tissues. The loci were often associated with a zone of surrounding hyalinized collagen similar to that surrounding some of the calcifications. DISCUSSION R u s h t o n 7 c o n s i d e r e d the cause o f r e g i o n a l o d o n t o d y s p l a s i a as either a single act o f d a m a g e with resulting s u b s e q u e n t a b n o r m a l i t i e s or as a c o n t i n u i n g insult. H o w e v e r , it has b e e n argued that a single act
o f d a m a g e w o u l d h a v e to o c c u r in utero and that it is difficult to e n v i s a g e such d a m a g e e x t e n d i n g f r o m the d e c i d u o u s to the p e r m a n e n t dentition, with the o c c a sional tooth in a q u a d r a n t r e m a i n i n g unaffected, s A c t i v a t i o n o f a latent virus in o d o n t o g e n i c epithel i u m has b e e n s u g g e s t e d as a cause for the disord e r v,9,10 but no virus particles h a v e b e e n identified.l 1, 12 L o c a l infection has also been cited, 13' 14 but the causal r e l a t i o n s h i p b e t w e e n infection and h y p o plasia is debatable.11, 15 S i m i l a r l y , local t r a u m a 1~ a6 has been i m p l i c a t e d . H o w e v e r , if t r a u m a is d e f i n i t e l y related to a specific d e f e c t in tooth d e v e l o p m e n t , then such a case p r o b a b l y s h o u l d not be c l a s s i f i e d as reg i o n a l o d o n t o d y s p l a s i a . 17 M e t a b o l i c or v i t a m i n deficiencies have also b e e n a d v a n c e d as causative factors, but it is u n l i k e l y that they w o u l d affect specific teeth and not others at s i m i l a r stages o f d e v e l o p m e n t . 16 R h e s u s i n c o m p a t i b i l i t y does not a p p e a r to be a conv i n c i n g cause, b e c a u s e d a m a g e o f a m o r e g e n e r a l i z e d nature w o u l d b e predicted. 3 Irradiation 7 can cause d a m a g e to d e v e l o p i n g teeth, 16 but in such cases a history w o u l d be available, and all the d e v e l o p i n g teeth in the treated area w o u l d be affected. T h e i n v o l v e m e n t o f s e l e c t e d teeth in r e g i o n a l o d o n t o d y s p l a s i a l e a v i n g a d j a c e n t teeth u n a f f e c t e d m a k e s r a d i a t i o n an u n l i k e l y cause. The s o m a t i c m u t a t i o n t h e o r y 7, 9 does not account for cases in w h i c h u n a f f e c t e d teeth h a v e d e v e l o p e d f r o m the distal e x t e n s i o n o f a m e s i a l l y aff e c t e d dental lamina, and in addition, w h e n teeth in both j a w s are affected, the chances o f m u l t i p l e m u t a tions in the s a m e patient are h i g h l y unlikely. 3 A her e d i t a r y factor can be d i s c o u n t e d b e c a u s e o f l a c k o f e v i d e n c e o f such a history in the cases r e p o r t e d to date. The p o s s i b i l i t y o f m a t e r n a l illness, drug therapy,
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Fig. 3. Periapical radiographs of lower anterior teeth showing reduced enamel and dentin tl with wide pulp chambers.
Fig. 4. Lateral skull radiograph demonstrating thin cortical margin of calvarium.
or both as etiologic factors is suggested by cases reported in children whose mothers had pneumonia 16 or toxemia 18 in pregnancy or who took diuretics, 19 tranquilizers, or hormones during the first trimester. 2~ Although a relationship between drugs and regional odontodysplasia has not been established, an increased awareness of the possible effects of such harmful agents is required. It is well established that a great variety of drugs taken during pregnancy can cause congenital malformations, for example, warfarin taken during the first trimester, 21 ethanol causing fetal alcohol syndrome, 22 cocaine producing congenital malformations, 22 aromatic retinoids used for severe skin disease, 23 and angiotensin-converting en-
zyme inhibitors for hypertension. 24 Children of women with epilepsy also appear to have increased evidence of congenital malformations; it is not certain whether this condition is due to drug therapy, but it seems likely. 22 A variety of abnormalities have been described that may accompany regional odontodysplasia. Vascular anomalies varying from a redness of the skin overlying the affected region that disappeared in later life to frank hemangioma have been reported, 3, 15, 25-28 and a local vascular defect or a local neural disorder (failure of neural crest cells to migrate) has been proposed as a factor in the etiology of the condition. 5 In support of the vascular etiology, changes similar to
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Fig. 5. Photomicrograph of gingival enlargement showing multiple foci of calcification (arrows) in richly cellular stroma, which includes odontogenic epithelium (E) surrounded by hyaline collagen, (Hematoxylineosin stain; original magnification x150.)
regional odontodysplasia have been shown to occur in experiments after occlusion of arterial supply to the region, e9,3~ Facial asymmetry caused by abnormal maxillary and mandibular development 31 and ipsilateral hypoplasia of the mid-face 15 have also been reported. DahlOf et al. 3a described a case of regional odontodysplasia with hydrocephalus. They proposed that localized brain damage may have affected the nerve supply of the developing jaws, thus contributing to the dental disorder. They quoted evidence that interference with the nerve supply of a developing tooth can produce dyplastic dentin. 33 Regional odontodysplasia has also been reported in a child with congenital bilateral talipes equinovarus 3~ and in association with orbital colobomas. 35 Cases have also been reported in a female child with hypophosphatasia, 36' in a male child with " h y p o c a l c a e m i a " shortly after birth, 37 and in a family with ectodermal dysplasia. 38 In this case the teeth that formed early were found to be affected to a greater degree than those that developed in later life, and this tendency has been confirmed in previously reported cases. 1~ 39 No history of the state of the deciduous.teeth could be obtained, but in those instances in which the deciduous teeth were lost before a diagnosis was made, evidence suggests that they were also affected. 12,2~ Nails are formed by invaginations of the embryonic epidermis at approximately 9 weeks' gestation and are completely formed by 12 weeks. 4~ Hairs initially appear in the third month in utero as cordlike epidermal
downgrowths into the mesenchyme. At birth the vault of the skull is unilaminar, and the tables and intervening dipl6e appear at approximately the fourth year41; by the sixth year the bones become three-layered with a dipl6e. 42 In this case the dolichocephalic calvarium was observed to be thin with poor evidence of dipl6e formation, especially on its superior aspect and near the occiput. It is unknown whether the various abnormalities reported to accompany regional odontodysplasia are purely incidental, but if a link exists between them, then the features in this case suggest that the causative factor would have to be operative in the first trimester of pregnancy. O f the predisposing factors reviewed, the suggestion of vascular disruption appears to be the most likely. Gingival involvement in regional odontodysplasia has been described infrequently, e' 17 and we have been unable to find other reports in which this was the patient's chief difficulty. Recognition of the gingival manifestations may therefore in certain cases assist in the early diagnosis of this uncommon disorder before the more usual presentation of caries and abscess formation. The combination of multiple foci of basophilic amorphous or lamellated calcifications associated with odontogenic epithelium distinguish the gingival enlargement from reactive hyperplasias and gingival fibromatosis. The histopathologic features in the gingiva closely resemble the calcifications associated with odontogenic epithelial nests seen characteristically in the follicles of involved teeth. 1, 11
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January 1996 REFERENCES 1. Crawford PJM, Aldred MJ. Regional odontodysplasia: a bibliography. J Oral Pathol 1989;18:251-63. 2. van der Wal JE, Rittersma J, Baart JA, van der Waal I. Regional odontodysplasia: report of three cases. Int J Oral Maxillofac Surg 1993;22:356-8. 3. Walton JL, Witkop CJ Jr, Walker PO. Odontodysplasia: report of three cases with vascular nevi overlying the adjacent skin of the face. ORAL SURG ORAL MED ORAL PATHOL 1978; 46:676-84. 4. Sadeghi EM, Ashrafi MH. Regional odontodysplasia: clinical, pathologic and therapeutic considerations. J Am Dent Assoc 1981;102:336-9. 5. Slootweg PJ, Meuwissen PRM. Regional odontodysplasia in epidermal nevus syndrome. J Oral Pathol 1985;14:256-62. 6. Diem K, Lentner C, editors. Documenta geigy, scientific tables. Macclesfield, England: Ciba-Geigy Ltd, 1981:308. 7. Rushton MA. Odontodysplasia: "ghost teeth." Br Dent J 1965;119:109-13. 8. Ferguson JW, Geary CPM. Regional odontodysplasia. Austral Dent J 1980;25:148-51. 9. Herman NG, Moss SJ. Odontodysplasia: report of case. J Dent Child 1977;44:52-4. 10. Bergman G, Lysell L, Pindborg JJ. Unilateral dental malformation. ORALSURGORALMED ORALPATHOL1963; 16:48-60. 11. Gardner DG, Sapp JP. Regional odontodysplasia. Oral Surg 1973;35:351-65. 12. Gibbard PD, Lee KW, Winter GB. Odontodysplasia. Br Dent J 1972;135:525-32. 13. Suher Th, Jump EB, Landis RL. Localised arrested tooth development. Oral Surg 1953;6:1305-14. 14. Hintz Ch S, Peters RA. Odontodysplasia. Oral Surg 1972;34: 744-50. 15. Schmid-Meier E. Unilateral odontodysplasia with ipsilateral hypoplasia of the mid-face. J Max-fac Surg 1982; 110:119-22. 16. Zegarelli EV, Kutscher AH, Applebaum E, Howell OA. Odonto-dysplasia. ORAL SURO ORAL MED ORAL PATHOL 1963;16:187-93. 17. Abrams AM, Groper J. Odontodysplasia, report of three cases. J Dent Child 1966;33:353-62. 18. Ferguson JW, Geary CPM. Regional odontodysplasia. Austral Dent J 1980;25:148-51. 19. Alexander WN, Lilly GE, Irby WB. Odontodysplasia. Oral Surg 1966;22:814-20. 20. Lustmann J, Klein H, Ulmansky M. Odontodysplasia. Report of two cases and review of the literature. Oral Surg 1975;39: 781-93. 21. Warkany J. Warfarin embryopathy. Teratology 1976;14:20510. 22. Farmer RE. Teratogenic drugs: an update. Adverse Drugs Reaction Bulletin 1993;161:607-10. 23. Dai WS, LaBraico JM, Stem RS. Epidemiology of isotetrinoin exposure during pregnancy. J Am Acad Dermatol 1992;26: 595-606. 24. Ban" M, Cohen M. ACE inhibitor fetopathy and hypocalvaria, the kidney-skull connection. Teratology 1991 ;44:485-95. 25. Kahn MA, Kinson RL. Regional odontodysplasia: case report
26. 27. 28.
29. 30. 31.
32. 33.
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37. 38.
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40. 41. 42.
with etiologic and treatment considerations. ORALSURGORAL MED ORAL PATHOL 1991;72:462-7. Fearne J, Williams DM, Brook AH. Regional odontodysplasia: a clinical and histological evaluation. J Int Assoc Dent Child 1986;17:21-5. Guzman R, Elliot MA, Rossia KM. Odontodysplasia in a paediatric patient: literature review and case report. Pediatric Dent 1990;12:45-8. Steiman HR, Cullen CL, Geist JR. Bilateral mandibular odontodysplasia with vascular nevus. Pediatric Dent 1991; 13: 303-6. Lumin M, Devore D. The etiology of odontodysplasia [Abstract]. J Dent Res 1976;55:B109. Kraus BS, Myers RE, Clark GR. Teratogenic effects of carotid ligation on the developing dentition of the rhesus monkey. Teratology 1969;2:163-72. Pandis N, Polido C, Bell WH. Regional odontodysplasia. A case associated with asymmetric maxillary and mandibular development. ORAL SURG ORAL MED ORAL PATHOL 1991; 72:492-6. Dahl6f G, Lindskog S, Theorell K, Ussisoo R. Concomitant regional odontodysplasia and hydrocephalus. ORAL SURG ORAL MED ORAL PATHOL 1987;63:354-7. Avery JK, Cox CF. Role of nerves in teeth relative to pain and dentinogenesis. In: Anderson DJ, Mathews B, editors. Pain in the trigeminal region. Amsterdam: North-Holland Pub Co, 1977: 137-48. Pruhs RJ, Simonsen CR, Sharma PS, Fodor B. Odontodysplasia. J Am Dent Assoc 1975;91:1057-66. Williams SA, High AS. Odontodysplasia associated with orbital coloboma. Br Dent J 1988;164:390-4. Russell K, Yacobi R. Generalised odontodysplasia concomitant with mild hypophosphatasia: a case report. Can Dent Assoc J 1993;59:187-90. Sibley LC, Zimmerman ER. Odontogenic dysplasia. Oral Surg 1962;15:1370-3. Gualandri V, Piattoni F, Orsini GB, Gueli MR, Polenghi M. Ectodermal dysplasia with severe odontodysplasia. Study of an affected family. Rev Chir Maxillofac 1988;89:102-5. Kinirons MJ, O'Brian FV, Gregg TA. Regional odontodysplasia: an evaluation of three cases based on clinical, microradiographic and histopathological findings. Br Dent J 1988; 165:136-9. ZaiasN. Embryologyofthehumannail. ArchDermatol 1963; 87:37-53. Williams PL, Warwick R, Dyson M, Bannister L, editor. Gray's Anatomy. London: Churchill Livingstone, 1989:91. Enlow DH Facial Growth. London, WB Saunders Co, 1990: 326, 936.
Reprint reqUests: K.B. Fanibunda, MDS, FDSRCS Department of Oral Surgery The Dental School University of Newcastle upon Tyne Newcastle upon Tyne, NE2 4BW United Kingdom
Regional odontodysplasia is an uncommon developmental dental disorder that may occasionally be accompanied by other abnormalities. A case is described in which the chief report was of a gingival enlargement arising in a female patient who also had dolichocephaly, thin calvarium, clinodactyly and transverse grooving of her fingernails, and a history of abnormal hair. Previously suggested etiologic factors and cases reported in association with other abnormalities are reviewed. (ORALSURGORALMED ORALPATHOLORALRADIOLENDOD1996;81:84-8)
R e g i o n a l o d o n t o d y s p l a s i a is a relatively u n c o m m o n c o n d i t i o n that u s u a l l y affects b o t h p r i m a r y and perm a n e n t dentitions. It occurs m o r e c o m m o n l y in f e m a l e patients and frequently i n v o l v e s the m a x i l l a r y teeth. 1 Clinically, the teeth a p p e a r discolored, h y p o plastic, and m a l f o r m e d , and the patient u s u a l l y has p a i n and s w e l l i n g c a u s e d b y p u l p a l and p e r i a p i c a l infection. R a d i o g r a p h i c a l l y , the teeth present a " g h o s t l i k e " i m a g e b e c a u s e o f the increased r a d i o l u c e n c y and thinness o f the e n a m e l and dentin and a w i d e pulp chamber. M i c r o s c o p i c a l l y , h y p o p l a s i a and h y p o m i n eralization are seen within the e n a m e l with large int e r g l o b u l a r areas in the dentin. The dental follicle m a y contain irregular calcifications. To date a p p r o x i m a t e l y 125 cases h a v e b e e n r e p o r t e d , and the dental features are w e l l d o c u m e n t e d . H o w e v e r , the cause is u n k n o w n , although several suggestions have b e e n a d v a n c e d . 2-5 This article reports an unusual case o f r e g i o n a l odo n t o d y s p l a s i a in w h i c h g i n g i v a l e n l a r g e m e n t was the presenting feature rather than the m o r e c o m m o n l y r e p o r t e d s y m p t o m s o f dental infection. CASE REPORT A 17-year-old white girl was referred by her dental practitioner for attention of a swelling of unknown duration situated in the left lower anterior region of her mouth. On general observation the patient was underweight for her age; she weighed 104 pounds (mean for age, 124.6 pounds6). Her height was within standard limits. On intraoral examination a gingival enlargement was noted on
aSenior Lecturer and Honorary Consultant, Department of Oral Surgery. bprofessor of Oral Pathology and Honorary Consultant, Newcastle upon Tyne, U.K. Department of Oral Pathology. Received for publication Feb. 15, 1995; returned for revision April 19, 1995; accepted for publication June 19, 1995. Copyright 9 1996 by Mosby-Year Book, Inc. 1079-2104/96/$5.00 + 0 7/14/67431 84
Fig. 1. Intraoral view of left lower quadrant showing discolored hypopiastic teeth accompanied by gingival enlargement. the labial and lingual aspects of the lower incisor to premolar region on the left side (Fig. t). The left lower central incisor, canine, and first premolar were discolored, pitted, and hypoplastic and gave the appearance of partial eruption because of the surrounding gingival enlargement. The lateral incisor in that quadrant was absent. The left lower first molar and the lower central incisor, lateral incisor, both premolars, and second molar on the right side were also affected by pitting and hypoplasia. The left lower second premolar and second molar were unaffected. Her fingers showed clinodactyly with ulnar or radial deviation of most digits. The nails on all her fingers had an increased curvature and exhibited transverse grooving (Fig. 2), which had been present throughout the patient's life. The nails on her toes were unaffected. Her stepparents reported that when the patient was adopted at 8 years of age, her hair was sparse, thin, and dull in color. However, the appearance had improved over the years. No other cranial or facial anomalies or abnormalities elsewhere were identifiable to link the presenting features with any syndrome. Because the patient was adopted, it was not possible to obtain a family
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Fig. 2. Nails demonstrate increased curvature and transverse grooving.
history, a history of the state of her deciduous teeth, or a history of any early birthmarks that may have disappeared later. Radiographic examination of the left lower central incisor, canine, first premolar, and the right lower central and lateral incisors revealed a reduced enamel and dentin thickness and wide pulp chambers (Fig. 3). No bony pathosis was seen associated with the roots of the teeth in the region of the swelling. Evidence of hypoplasia but not wide pulp chambers was also confirmed in the remaining clinically affected teeth mentioned previously. The root lengths of the left lower central incisor, canine, and first premolar were reduced. The unerupted right lower third molar showed features of "mulberry cusps." A lateral skull view demonstrated dolichocephaly and a relatively thinner than standard thickness of the cortical margins of the calvarium together with a pronounced parieto-occipital suture line (Fig. 4). In view of the presenting symptoms, the gingival enlargement was trimmed under local analgesia. Histopathologic examination showed chronically inflamed hyperplastic gingiva deep to which were numerous aggregates of rich cellular tissue containing multiple loci of basophilic amorphous or lamellated calcification (Fig. 5), Some of the lamellated calcifications were surrounded by a hyaline stroma with diffuse areas of calcification or were intimately associated with plump, fibroblast-like cells. Rests of odontogenic epithelium were present within the foci and surrounding tissues. The loci were often associated with a zone of surrounding hyalinized collagen similar to that surrounding some of the calcifications. DISCUSSION R u s h t o n 7 c o n s i d e r e d the cause o f r e g i o n a l o d o n t o d y s p l a s i a as either a single act o f d a m a g e with resulting s u b s e q u e n t a b n o r m a l i t i e s or as a c o n t i n u i n g insult. H o w e v e r , it has b e e n argued that a single act
o f d a m a g e w o u l d h a v e to o c c u r in utero and that it is difficult to e n v i s a g e such d a m a g e e x t e n d i n g f r o m the d e c i d u o u s to the p e r m a n e n t dentition, with the o c c a sional tooth in a q u a d r a n t r e m a i n i n g unaffected, s A c t i v a t i o n o f a latent virus in o d o n t o g e n i c epithel i u m has b e e n s u g g e s t e d as a cause for the disord e r v,9,10 but no virus particles h a v e b e e n identified.l 1, 12 L o c a l infection has also been cited, 13' 14 but the causal r e l a t i o n s h i p b e t w e e n infection and h y p o plasia is debatable.11, 15 S i m i l a r l y , local t r a u m a 1~ a6 has been i m p l i c a t e d . H o w e v e r , if t r a u m a is d e f i n i t e l y related to a specific d e f e c t in tooth d e v e l o p m e n t , then such a case p r o b a b l y s h o u l d not be c l a s s i f i e d as reg i o n a l o d o n t o d y s p l a s i a . 17 M e t a b o l i c or v i t a m i n deficiencies have also b e e n a d v a n c e d as causative factors, but it is u n l i k e l y that they w o u l d affect specific teeth and not others at s i m i l a r stages o f d e v e l o p m e n t . 16 R h e s u s i n c o m p a t i b i l i t y does not a p p e a r to be a conv i n c i n g cause, b e c a u s e d a m a g e o f a m o r e g e n e r a l i z e d nature w o u l d b e predicted. 3 Irradiation 7 can cause d a m a g e to d e v e l o p i n g teeth, 16 but in such cases a history w o u l d be available, and all the d e v e l o p i n g teeth in the treated area w o u l d be affected. T h e i n v o l v e m e n t o f s e l e c t e d teeth in r e g i o n a l o d o n t o d y s p l a s i a l e a v i n g a d j a c e n t teeth u n a f f e c t e d m a k e s r a d i a t i o n an u n l i k e l y cause. The s o m a t i c m u t a t i o n t h e o r y 7, 9 does not account for cases in w h i c h u n a f f e c t e d teeth h a v e d e v e l o p e d f r o m the distal e x t e n s i o n o f a m e s i a l l y aff e c t e d dental lamina, and in addition, w h e n teeth in both j a w s are affected, the chances o f m u l t i p l e m u t a tions in the s a m e patient are h i g h l y unlikely. 3 A her e d i t a r y factor can be d i s c o u n t e d b e c a u s e o f l a c k o f e v i d e n c e o f such a history in the cases r e p o r t e d to date. The p o s s i b i l i t y o f m a t e r n a l illness, drug therapy,
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Fig. 3. Periapical radiographs of lower anterior teeth showing reduced enamel and dentin tl with wide pulp chambers.
Fig. 4. Lateral skull radiograph demonstrating thin cortical margin of calvarium.
or both as etiologic factors is suggested by cases reported in children whose mothers had pneumonia 16 or toxemia 18 in pregnancy or who took diuretics, 19 tranquilizers, or hormones during the first trimester. 2~ Although a relationship between drugs and regional odontodysplasia has not been established, an increased awareness of the possible effects of such harmful agents is required. It is well established that a great variety of drugs taken during pregnancy can cause congenital malformations, for example, warfarin taken during the first trimester, 21 ethanol causing fetal alcohol syndrome, 22 cocaine producing congenital malformations, 22 aromatic retinoids used for severe skin disease, 23 and angiotensin-converting en-
zyme inhibitors for hypertension. 24 Children of women with epilepsy also appear to have increased evidence of congenital malformations; it is not certain whether this condition is due to drug therapy, but it seems likely. 22 A variety of abnormalities have been described that may accompany regional odontodysplasia. Vascular anomalies varying from a redness of the skin overlying the affected region that disappeared in later life to frank hemangioma have been reported, 3, 15, 25-28 and a local vascular defect or a local neural disorder (failure of neural crest cells to migrate) has been proposed as a factor in the etiology of the condition. 5 In support of the vascular etiology, changes similar to
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Fig. 5. Photomicrograph of gingival enlargement showing multiple foci of calcification (arrows) in richly cellular stroma, which includes odontogenic epithelium (E) surrounded by hyaline collagen, (Hematoxylineosin stain; original magnification x150.)
regional odontodysplasia have been shown to occur in experiments after occlusion of arterial supply to the region, e9,3~ Facial asymmetry caused by abnormal maxillary and mandibular development 31 and ipsilateral hypoplasia of the mid-face 15 have also been reported. DahlOf et al. 3a described a case of regional odontodysplasia with hydrocephalus. They proposed that localized brain damage may have affected the nerve supply of the developing jaws, thus contributing to the dental disorder. They quoted evidence that interference with the nerve supply of a developing tooth can produce dyplastic dentin. 33 Regional odontodysplasia has also been reported in a child with congenital bilateral talipes equinovarus 3~ and in association with orbital colobomas. 35 Cases have also been reported in a female child with hypophosphatasia, 36' in a male child with " h y p o c a l c a e m i a " shortly after birth, 37 and in a family with ectodermal dysplasia. 38 In this case the teeth that formed early were found to be affected to a greater degree than those that developed in later life, and this tendency has been confirmed in previously reported cases. 1~ 39 No history of the state of the deciduous.teeth could be obtained, but in those instances in which the deciduous teeth were lost before a diagnosis was made, evidence suggests that they were also affected. 12,2~ Nails are formed by invaginations of the embryonic epidermis at approximately 9 weeks' gestation and are completely formed by 12 weeks. 4~ Hairs initially appear in the third month in utero as cordlike epidermal
downgrowths into the mesenchyme. At birth the vault of the skull is unilaminar, and the tables and intervening dipl6e appear at approximately the fourth year41; by the sixth year the bones become three-layered with a dipl6e. 42 In this case the dolichocephalic calvarium was observed to be thin with poor evidence of dipl6e formation, especially on its superior aspect and near the occiput. It is unknown whether the various abnormalities reported to accompany regional odontodysplasia are purely incidental, but if a link exists between them, then the features in this case suggest that the causative factor would have to be operative in the first trimester of pregnancy. O f the predisposing factors reviewed, the suggestion of vascular disruption appears to be the most likely. Gingival involvement in regional odontodysplasia has been described infrequently, e' 17 and we have been unable to find other reports in which this was the patient's chief difficulty. Recognition of the gingival manifestations may therefore in certain cases assist in the early diagnosis of this uncommon disorder before the more usual presentation of caries and abscess formation. The combination of multiple foci of basophilic amorphous or lamellated calcifications associated with odontogenic epithelium distinguish the gingival enlargement from reactive hyperplasias and gingival fibromatosis. The histopathologic features in the gingiva closely resemble the calcifications associated with odontogenic epithelial nests seen characteristically in the follicles of involved teeth. 1, 11
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Reprint reqUests: K.B. Fanibunda, MDS, FDSRCS Department of Oral Surgery The Dental School University of Newcastle upon Tyne Newcastle upon Tyne, NE2 4BW United Kingdom